ArticleBMJ case reports2023
Secondary haemophagocytic lymphohistiocytosis in a patient with new-onset systemic lupus erythematosus: the challenges of timely diagnosis and successful treatment.
Article in BMJ case reports, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 3 citations in OpenAlex.
- Macrophage activation syndrome in systemic lupus erythematosus: risk factors and outcomes.Clinical rheumatology · 2026Article
- Cytomegalovirus-induced hemophagocytic lymphohistiocytosis revealing systemic lupus erythematosus: a case report.Annals of medicine and surgery (2012) · 2026Article
- Long-Term Treatment With Emapalumab in an Adult Patient With Refractory Hemophagocytic Lymphohistiocytosis and Systemic Lupus Erythematosus: A Case Report.Case reports in hematology · 2026Article
- Kidney Dysfunction and Pathology in the Setting of Hemophagocytic Lymphohistiocytosis.Kidney international reports · 2024Article
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Haemophagocytic lymphohistiocytosis (HLH) is an immune-mediated disease driven by abnormal macrophage activation and regulatory cell dysfunction. HLH can be primary due to genetic mutations or secondary due to infection, malignancy or autoimmune conditions. We describe a woman in her early 30s who developed HLH while being treated for newly diagnosed systemic lupus erythematosus (SLE) complicated by lupus nephritis as well as concomitant cytomegalovirus (CMV) reactivation from a dormant infection. The trigger for this secondary form of HLH may have been either aggressive SLE and/or CMV reactivation. Despite prompt treatment with immunosuppressive therapies for SLE consisting of high-dose corticosteroids, mycophenolate mofetil, tacrolimus, etoposide for HLH and ganciclovir for CMV infection, the patient developed multiorgan failure and passed away. We demonstrate the difficulty in identifying a specific cause for secondary HLH when multiple conditions are present (SLE and CMV) and the fact that, despite aggressive treatment for both conditions, the mortality for HLH remains high.
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Registered trials
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